Primary Diffuse Large B-Cell Lymphoma of Ovary: Report of Cases and Literature Review

Case Report

Ann Hematol Oncol. 2022; 9(3): 1397.

Primary Diffuse Large B-Cell Lymphoma of Ovary: Report of Cases and Literature Review

Zhang XF¹, Diao XL², Zhao YT³, Wang GR4, Wang SZ¹* and Zhu L¹*

1Department of Obstetrics and Gynecology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

2Department of Pathology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

3Department of Clinical Chemical Examination, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

4Department of Hematology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

*Corresponding author: Wang SZ, Department of Obstetrics and Gynecology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

Zhu L, Department of Obstetrics and Gynecology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China

Received: May 30, 2022; Accepted: June 28, 2022; Published: July 05, 2022


Objective: Our study aimed to review the characters of primary diffuse large B-cell lymphoma (DLBCL) of ovary and provide recommendations of management for this rare disease.

Methods: We presented 4 cases of primary ovarian DLBCL (PODLBCL) from Beijing Chaoyang Hospital between 2003 and 2021. Furthermore, we searched PubMed and Web of Science database for literature published between 1998 and 2021.A total of 16 articles included 24 cases with histopathologically confirmed PODLBCL and detailed evaluation and follow-up were reviewed.

Results: The median age of patients with PODLBCL was 43y (5-73y). The main clinical symptoms were pelvic mass, abdominal pain, followed by irregular vaginal bleeding and urinary incontinence. 25% patients had bilateral ovarian tumors, and 61.1% of unilateral tumors were in the left ovary. Cancer antigen 125 (CA125) and serum lactate dehydrogenase (LDH) were usually elevated to varying degrees. Most cases were diagnosed by surgery and presented in early stage. Treatments included surgery, chemotherapy and monoclonal antibody therapy. There was no evidence of recurrence within a median follow-up time of 20 months (range 5-72 months) in 79.1% (19/21) of patients.

Conclusions: PODLBCL had no significantly specific character to distinguish from ovarian epithelial malignant tumors. The prognosis of patients with PODLBCL was usually good. We proposed that chemotherapy combined with monoclonal antibody therapy may be the first-line treatment for PODLBCL, and surgical resection of the tumor may be avoided. The key problem is how to make an early preoperative diagnosis. Therefore, more case reports and institutional studies are needed to confirm these conclusions.

Keywords: Diffuse Large B-Cell Lymphoma; Primary Ovarian Lymphoma; Management


DLBCL: Primary Diffuse Large B-Cell Lymphoma; PODLBCL: Primary Ovarian DLBCL; POL: Primary Ovarian Lymphoma; CA125: Cancer Antigen 125; LDH: Lactate Dehydrogenase; NHL: Non- Hodgkin Lymphoma; PLFGT: Primary Lymphoma of the Female Genital Tract; CEA: Carcinoembryonic Antigen; LCA: Leukocyte Common Antigen; R-CHOP: Cyclophosphamide, Vincristine, Doxorubicin, Prednisone, and Rituximab; CNS: Central Nerve System; GCB: Germinal Center B-Cell-Like Lymphoma; IPI: The International Prognostic Index; ECOG: Eastern Cooperative Oncology Group; OS: Overall Survival; NCCN: National Comprehensive Cancer Network; PD-L1: Programmed Cell Death Ligand-1.


Although reports of lymphoma involving the reproductive tract have become more common recently, the initial presentation of lymphoma as an ovarian mass is rare and is called as primary ovarian lymphoma (POL), accounting for 0.5% of all non-hodgkin lymphoma(NHL)cases and 1.5% of all ovarian tumors [1]. Nasioudis et al. conducted the largest cohort study on primary lymphoma of the female genital tract (PLFGT) and showed that POL accounts for 37% of all PLFGT cases [2]. Diffuse large B-cell lymphoma is the most common type of POL [3] and the prognosis of primary ovarian DLBCL has been debated by researchers. According to literatures, there is no standard therapeutic management for PODLBCL because of its rarity [4]. Here, we reported 4 cases of PODLBCL in our institution andreviewed the cases in literatures, aimingat analyzing the clinical characteristics, prognostic factors, and treatment outcome of patients with PODLBCL.

Case 1

A 57-year-old post-menopausal womanvisited our hospital with complaints of urination frequency for a month and persistent high fever (>38.5°C) for 2 weeks. Past history is nothing remarkable. Physical examination revealed a large solid pelvic mass and bilateral lower extremity edema. Routine blood examination revealed severe macrocytic anemia and thrombocytopenia. An elevated C-reactive protein level at 91 mg/L was also noted. Serum tumor markers were positive forCA125 (217 U/mL). Laboratory examination revealed highly elevated LDH (543 U/L). Other laboratory examinations were all within normal limits. Ultrasonography showed a heterogeneous solid mass measuring 14×11×10 cm in the pelvis and the right urinary tract compressed by the large mass. No lymphadenopathy was noted. Bone marrow biopsy and aspiration were normal. After transfusion with washed red blood cells and platelets, an exploratory laparotomy was performed. Approximately 500 ml dark red serous ascitic fluid and a 15cm multinodular solid mass were identified in the peritoneum. The normal anatomical structure of the Douglaspouch had disappeared. Histologic examination of anintraoperative frozen section from the mass showed a malignant neoplasm. As a result, a debulking surgery of the tumor was completed with a right salpingo-oophorectomy, partial omentectomy and appendectomy. Pathological examination revealed DLBCL of the right ovary (Figure 1). According to the Ann Arbor system, the patient had stage BE disease. The patient received 6 courses of R-CHOP (cyclophosphamide, vincristine, doxorubicin, prednisone, and rituximab). She was also administered intrathecal methotrexate (15 mg on day 2) to prevent central nerve system (CNS) relapse. Follow-up to 60 months after surgery showed no evidence of recurrence.